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布卢姆综合征成纤维细胞培养物的致断裂活性。

Clastogenic activity from Bloom syndrome fibroblast cultures.

作者信息

Emerit I, Cerutti P

出版信息

Proc Natl Acad Sci U S A. 1981 Mar;78(3):1868-72. doi: 10.1073/pnas.78.3.1868.

Abstract

Media from cultures of fibroblasts of six patients with the autosomal recessive disease Bloom syndrome (BS) and from four normal fibroblast strains were analyzed for clastogenic activity towards phytohemagglutinin-stimulated human blood lymphocytes from healthy donors. Clastogenic activity was detected in concentrated ultrafiltrates of media from all six BS strains but none of the normal fibroblast strains. The frequencies of chromosomal aberrations that were induced depended on the concentration of the ultrafiltrates. Addition of bovine superoxide dismutase to the blood lymphocyte cultures strongly suppressed the clastogenic potency of the ultrafiltrates. Unconcentrated conditioned BS media were inactive. From the pore size of the ultrafilters and Sephadex G-10 chromatography it is concluded that the clastogenic material is in the molecular weight range of 1000 to 10,000. The concentrated ultrafiltrates of BS culture media also possessed the capacity to induce sister chromatid exchanges in normal human blood lymphocytes, but with relatively low efficiency. On the basis of these results and by analogy to certain collagen diseases such as systemic lupus erythematosus, we speculate that BS cells are deficient in the detoxification of active oxygen species.

摘要

对6名患有常染色体隐性疾病布卢姆综合征(BS)患者的成纤维细胞培养物的培养基,以及4种正常成纤维细胞株的培养基,进行了针对来自健康供体的植物血凝素刺激的人血淋巴细胞的致断裂活性分析。在所有6种BS细胞株的培养基浓缩超滤物中均检测到致断裂活性,但正常成纤维细胞株的培养基中均未检测到。诱导产生的染色体畸变频率取决于超滤物的浓度。向血淋巴细胞培养物中添加牛超氧化物歧化酶可强烈抑制超滤物的致断裂能力。未浓缩的BS条件培养基无活性。根据超滤器的孔径和葡聚糖G - 10色谱分析得出,致断裂物质的分子量范围在1000至10000之间。BS培养基的浓缩超滤物也具有在正常人血淋巴细胞中诱导姐妹染色单体交换的能力,但效率相对较低。基于这些结果,并与某些胶原疾病如系统性红斑狼疮进行类比,我们推测BS细胞在活性氧解毒方面存在缺陷。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c67c/319236/aa3541eb8b48/pnas00654-0583-a.jpg

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